Background: The hemolytic anemias are group of disorders characterized by increased destruction of RBC while bone marrow compensating for hemolysis by increased erythropoisis. Hemolytic anemias can be due to a corpuscular defect (mainly congenital) or due to an abnormal hemolytic mechanism (extrinsic abnormality).Among the congenital causes, Hemoglobinopathies and thalassemia constitute a major proportion.

Methods: A prospective study was carried out among 135 patients of congenital hemolytic anemia in IMS and SUM Hospital, Bhubaneswar, Odisha.  a tertiary care teaching hospital at eastern India for a period of one year. Detailed information on demographic pattern, clinical profile and hematological parameters were assessed.

Results: Out of the total 135 patients evaluated, it was found that the most common cause of congenital hemolytic anemia was sickle cell trait (35.5%)  followed by sickle cell disease (30.4%)  beta Thalassemia trait(17.9%), beta thalassemia major (7.4%). The mean hemoglobin was found to be lowest in beta thalassemia major (6.10mg/dl). The most common presenting symptom was hemolytic facies(53.33%) followed by growth retardation (49.62%). Most patients were residing from the district of Nayagarh (46%)

Conclusion: The incidence of sickle cell trait is relatively higher in comparison to other causes of hemoglobinopathies and is a major health problem in eastern area of the country.

Keywords: Beta thalassemia,  Congenital hemolytic anaemia,  Sickle cell trait.

1. Surhone LM, Tennoe MT, Henssonow SF. Congenital Hemolytic Anemia. Saarbrücken, Germany: VDM Verlag Dr. Müller; 2010.
2. Balgir RS. Control and prevention of the genetic load of hemoglobinopathies in India. Natl Med J India. 1999;12: 234-238
3. Varawalla NY, Old JM, Sarkar R, Venkatesan R, Weatherall DJ. The spectrum of beta thalassemia mutations on the Indian subcontinent: the basis for prenatal diagnosis. Brit J Hematol. 1991;78(2):242-7.
4. Ghai OP. Gupta P, Paul VK. Essential Pediatrics. 6th ed. New Delhi: Interprint; 2004. Chapter 12, Hematological disorders; p.100-101.
5. La Nasa G, Caocci G, Argiolu F et al: Unrelated donor stem cell transplantation in adult patients with thalassemia. Bone Marrow Transplant. 2005 Dec;36(11):971-5.
6. Chatterjee et al: Quoted by Chatterjee JB (1970). Proc XII, Cong of Indian Soc. Haem, 1970.
7. Agarwal S, Gupta A, Gupta UR, Sarwai S, Phadke S, Agarwal SS. Prenatal diagnosis in Beta-Thalassaemia: An Indian experience. Fetal Diagn Ther. 2003;18:328–32
8. Agarwal MB, Mehta BC. Genotypic analysis of symptomatic thalassemia syndromes -A study of 292 unrelated cases from Bombay. J Postgrad Med 1982;28:1-3.
9. Agarwal MB, Mehta BC. Symptomatic beta thalassemia trait-A study of 143 cases. J Postgrad Med 1982;28:4-8.
10. Weatherall DJ, Clegg JB. Thalassemia - a global health problem. Nat Med. 1996;2:847-9.
11. Deyde VM, Lo BB, Aw T. Hb hope/HbS and HbS/ß- thal double compound heterozygosity in a Mauritanian family: clinical and biochemical studies. Ann Hematol. 2003;82:423.
12. Cunningham MJ. Update on thalassemia: Clinical care and complications. Pediatr Clin North Am. 2008;55:447-60.
13. Bernard SS. Genetic counseling for thalassemia in the islamic republic of Iran. Johns Hopkins University Press. 2009;52(3):364-76.

Dr Braja Kishore Behera

Assistant Professor, Department of pediatrics, IMS & SUM Hospital, Siksha O Anusandhan University, K8, kalinga nagar, Bhubaneswar- 751003, Odisha, India

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